Major Aortopulmonary Collateral Arteries Research Articles

Association of right aortic arch and agenesis of ductus arteriosus in prenatal tetralogy of Fallot spectrum and its clinical implications.

In our center, we observed an increased frequency of right aortic arch (RAA) with an agenesis of the ductus arteriosus (ADA) in prenatally diagnosed tetralogy of Fallot (ToF) and its variations. This study aimed to determine whether there is an association of RAA and ADA in fetuses with ToF. Distribution of genetic anomalies and impact on postnatal outcome were further evaluated. Single-center retrospective observational study including pregnancies with prenatal diagnosis of ToF from 2010 to 2023. All cases were subdivided into ToF with pulmonary stenosis (PS) and pulmonary atresia (PA). Clinical and echocardiographic databases were reviewed for pregnancy outcome, genetic anomalies, and postnatal course. The cohort included 169 cases, 124 (73.4%) with ToF/PS and 45(26.6%) with ToF/PA. Agenesis of the ductus arteriosus was significantly associated with RAA in both subtypes of ToF (p=0.001) compared to left aortic arch and found in 82.5% (33/40) versus 10.7% (9/84) of fetuses with ToF/PS and in 57.1% (8/14) versus 12.9% (4/31) of fetuses with ToF/PA. In both ToF/PS and ToF/PA, RAA/ADA versus RAA/patent DA revealed a significantly higher risk for the presence of genetic abnormalities, especially microdeletion 22q11.2, major aorto-pulmonary collateral arteries and a shorter time to complete surgical repair. We demonstrated a significantly increased frequency of RAA/ADA in patients with prenatally diagnosed ToF. Although this association revealed no significant impact on overall survival, the prenatal detection of RAA/ADA has implications for counseling, genetic evaluation and postnatal management.

Pulmonary flow-study can predict in-hospital prognosis of unifocalization and corrective repair of pulmonary atresia/ventricular septal defect with major aortopulmonary collateral arteries

ObjectivesWith the development of perioperative treatment, the results of the unifocalization and corrective repair of pulmonary atresia/ventricular septal defect with major aortopulmonary collateral arteries have been significantly improved. However, the in-hospital recovery is quite different individually. Therefore, it is essential to find prognostic indicators to avoid unsatisfactory recovery. DesignThis was a case-control study. SettingThe study was conducted in the national center for cardiovascular diseases in China. ParticipantsPediatric patients between 2014 and 2022. InterventionsNone. Measurements & main resultsA total of 19 patients were included. The possible prognostic indicators included were commonly used clinical data. Unsatisfactory postoperative recovery was defined as mechanical ventilation≥ 7 days and/or in-hospital death. Satisfactory postoperative recovery was defined as mechanical ventilation<7 days and survival at discharge. We separated patients into two groups and compared the peri-operative data through univariable analysis. There were 8 patients in unsatisfactory recovery group (including 1 death) and 11 patients in satisfactory recovery group. Among all the possible prognostic indicators, through univariable analysis, pulmonary arterial pressure in pulmonary flow study was statistically different (p = 0.027 < 0.05). The ROC curve showed that the area under curve and cut-off values in predicting satisfactory recovery were 0.841 and 22 mmHg; the corresponding sensitivity and specificity were 100% and 72.7%. There was no statistical difference between the two groups in ventricular septal fenestration and pulmonary hypertension targeting drugs. ConclusionA pulmonary arterial pressure <22 mmHg in pulmonary flow study may avoid unsatisfactory in-hospital recovery after unifocalization and corrective repair of pulmonary atresia/ventricular septal defect with major aortopulmonary collateral arteries.

Bridge to Surgery with Pulmonary Valve Perforation and Right Ventricular Outflow Tract Stenting in an Infant with PA-VSD

Pulmonary atresia (PA) with ventricular septal defect (VSD) is a rare congenital heart disease. Blood flow to pulmonary arteries is provided by patent ductus arteriosus or major aorto-pulmonary collateral arteries (MAPCAs). Palliative interventions are required to maintain the blood supply to the pulmonary arteries in order to achieve for the complete surgical repair. In this case report, we present a patient who underwent pulmonary valve perforation and right ventricular outflow tract (RVOT) stenting as an alternative to a systemic-pulmonary artery shunt (SPS). Subsequently, surgical repair was performed ten months later.

Aortic valve replacement in a 41-year-old woman with uncorrected tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries: a case report

BackgroundTetralogy of Fallot (TOF) is a complex cyanotic congenital heart disease. As most patients with TOF undergo palliative or radical surgical repair during childhood, cardiac surgery under cardiopulmonary bypass (CPB) for adult survivors with unrepaired TOF is exceedingly rare.Case presentationA 41-year-old woman with unrepaired TOF, pulmonary atresia (PA), and major aortopulmonary collateral arteries (MAPCAs) developed acute infectious endocarditis (IE). As vegetation gradually increased despite intravenous antibiotic administration, she was scheduled for urgent aortic valve replacement under CPB. Pulmonary blood flow was primarily provided by the MAPCAs originating from the descending aorta. Intra-aortic balloon occlusion for MAPCAs was performed to ensure a bloodless surgical field. Aortic valve replacement was successful.ConclusionAn adult with uncorrected TOF developed acute IE and subsequently had successful cardiac surgery under CPB. Understanding TOF physiology with PA and MAPCAs, particularly pulmonary blood flow through MAPCAs, is crucial.

Outcomes after surgical repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: a series of 104 cases

Objective: To examine the early and midterm surgical outcome of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCA) using revised surgical strategies. Methods: A retrospective analysis of clinical data, surgical methods, and follow-up results was performed of 104 cases of PA/VSD/MAPCA in Department of Cardiovascular Surgery, Guangzhou Women and Children's Medical Center from January 2017 to September 2022. There were 55 males and 49 females, aged (M(IQR)) 33.9(84.0) months (range: 0.5 to 209.6 months) at the first surgical procedures. The anatomical classification included 89 cases of type B and 15 cases of type C. The number of major aortopulmonary collateral arteries was 4.2 (3.0) (range: 1 to 8). The Kaplan-Meier method was used for survival estimation. Results: In the first stage of surgery, 50 patients underwent a complete primary repair, 12 patients underwent partial repair, 32 patients underwent palliative right ventricular-pulmonary artery connection, and only 10 patients chose the Blalock-Taussig shunt. There were 10 cases of early death. In the second stage, 14 patients underwent complete repair and 4 patients underwent partial repair with no early death. The interval between the two surgeries was 19 (10) months (range: 9 to 48 months). Finally, during the 40 (34) months follow-up period, a total of 64 patients were complete repair and the right/left ventricular pressure ratio after complete repair was 0.63±0.16 (range: 0.36 to 1.00). Survival analysis showed that survival rates at 1 and 5 years after first-stage surgery were both 89.4% (95%CI: 83.5% to 95.3%). At 28 (34) months (range: 1 to 67 months) of follow-up after complete repair, the survival analysis showed that the survival rates at 1 and 5 years were both 95.2% (95%CI: 89.9% to 100%). Conclusions: Using combined approaches tailored to individual patients and optimized unifocalization strategy, the complete repair rate at one stage and the cumulative complete repair rate at 5 years improved significantly with a lower right/left ventricular pressure ratio and satisfactory early and intermediate survival.

Clarifying the Pulmonary Arterial Morphology and Pulmonary Blood Supply in Patients with Tetralogy of Fallot and Pulmonary Atresia on Computed Tomography Angiography

Aim. The present study sought to characterize the pulmonary arterial morphology and pulmonary blood supply in patients of tetralogy of Fallot and pulmonary atresia (TOF-PA) on CT angiography. Materials and Methods. We retrospectively reviewed our departmental database to identify patients with TOF-PA evaluated using CT angiography. The images were analysed to detect the presence of main and branch pulmonary arteries and pulmonary arterial confluence, presence of major aortopulmonary collateral arteries (MAPCAs), laterality and relation with pulmonary arterial morphology, and presence of patent arterial duct and associated intra- and extracardiac anomalies. Results. TOF-PA was identified in 177 patients (114 (64.4%) males; median age: 9 months). Pulmonary arteries were confluent in 142/177 (80.2%) patients. According to Somerville classification, type II pulmonary atresia was the most frequent pattern seen in 127/177 (71.8%). Based on McGoon’s ratio, pulmonary arteries were adequate for surgery in 123/177 (69.5%) patients. Patent arterial duct was present in 84/177 (47.5%) patients while MAPCAs were present in 124 (70.1%) patients, of which 72/124 (58.1%) patients had at least 1 essential MAPCA supplying either lung. According to Congenital Heart Surgeons’ Society classification, type B pulmonary arterial anatomy was the most prevalent, seen in 103/177 (57.6%) patients. Conclusion. TOF-PA is associated with marked morphologic variability in the pulmonary arterial arborization to supply the lungs. Cardiac CT angiography can accurately delineate the pulmonary arterial morphology, sources of pulmonary blood supply, and associated cardiovascular anomalies in patients with TOF-PA which aids in planning appropriate surgical management including decisions regarding the need for unifocalization of MAPCAs.

Pulmonary atresia and ventricular septal defect: How accurate is the fetal echocardiography, and do the major aortopulmonary collateral arteries matter?

To assess the accuracy of prenatal echocardiography in defining pulmonary vasculature in pulmonary atresia with VSD (PAVSD). The second aim is to compare the perinatal and postnatal outcomes of different pulmonary blood supply types. The cases prenatally diagnosed with PAVSD between 2017 and 2022 in a single tertiary fetal medicine center were identified on the electronic database. Fetal echocardiography reports and images were reviewed retrospectively. Postnatal outcomes were acquired from the hospital records of relevant pediatric cardiology and cardiovascular surgery clinics. Fetal echocardiography results were compared with postnatal results. Perinatal and postnatal outcomes were compared between the different pulmonary vascular supply types. Among the 24 PAVSD cases, six were diagnosed with major aortopulmonary collateral arteries (MAPCA) dependent, eleven were diagnosed with ductus arteriosus (DA) dependent pulmonary supply, and seven were diagnosed with double pulmonary supply (MAPCA + DA) on prenatal echocardiography. Seventeen cases were live-born and have undergone postnatal investigations. Fetal echocardiography was 88.2% accurate about the type of pulmonary supply. The accuracy of fetal echocardiography regarding pulmonary vascular anatomy was 82.3%. Postoperative survival was 69.2%. Mortality before surgery and postoperative survival did not differ between pulmonary supply groups. Survival was impaired by the extracardiac anomalies. The need for early interventions was significantly higher in the DA group. Pulmonary vascularization in PAVSD can be defined precisely on fetal echocardiography. The source of pulmonary blood supply does not impact postnatal short-term outcomes significantly but it impacts the management. The associated anomalies highly contribute to postnatal mortality.

Clinical effects of major aortopulmonary collateral arteries in term neonates diagnosed with transposition of the great arteries.

Transposition of the great arteries is a severe CHD that affects term neonates. The presence of major aortopulmonary collateral arteries in neonatal transposition of the great arteries patients is rare. This study investigated the clinical and haemodynamic implications of the presence of major aortopulmonary collateral arteries in neonatal transposition of the great arteries patients who underwent an arterial switch operation. The study was a retrospective analysis conducted on neonates diagnosed with transposition of the great arteries who underwent arterial switch operation within the period from 1 May 2020 to 1 January 2023 at two high-patient-volume paediatric cardiac surgery centres in Turkey. The patients' demographic characteristics, echocardiographic features, and clinical data were analysed. Additionally, the possible clinical effects of the presence of major aortopulmonary collateral arteries were statistically evaluated. Two hundred cases of neonatal transposition of the great arteries were included in this study, with 55% of the cases male. All the patients underwent arterial switch operation. The median age at the time of arterial switch operation was 5 days (interquartile range 3-7), with a median weight of 3,100 g (interquartile range 2,900-3,400). The median pre-operative saturation level was 76% (interquartile range 70-82%). Prior to arterial switch operation, 32 patients underwent balloon atrial septostomy.In all the patients, the interatrial septum was checked to determine if the atrial septum was intact. A patent foramen ovale (≤ 3 mm) was found in 112 patients, and a non-restrictive atrial septal defect (> 3 mm) was found in 88. Forty-eight patients had ventricular septal defects, and 72 had coronary anomalies. Major aortopulmonary collateral arteries were found in 4 patients pre-operatively and in 12 patients after arterial switch operation (echocardiography, n = 8; angiography, n = 4). Of the patients with post-operative detection of cumulative number of major aortopulmonary collateral arteries were on post-operative day 1 in 2 patients, on post-operative day 3 in 5 patients, on post-operative day 7 in 6 patients, and on post-operative day 14 in 11 patients.Transcatheter closure was performed in 3 cases due to recurrent extubation failure. Major aortopulmonary collateral artery shrinkage was observed in one case under medical treatment. The length of paediatric cardiac intensive care unit stay (10 days versus 8 days; p < 0.005), mechanical ventilator time (4 days versus 2 days; p = 0.02), and inotrope use time (5 days versus 3 days; p = 0.04) were higher in the major aortopulmonary collateral artery cases than patients without major aortopulmonary collateral artery. Major aortopulmonary collateral arteries are frequent in transposition of the great arteries patients and may have clinical effects. The presence of major aortopulmonary collateral arteries should be investigated in patients who do not have a favourable post-operative course after arterial switch operation.

Retracted: Verifying the Usefulness of Pulmonary Blood Flow Studies in the Correction of Pulmonary Atresia and Ventricular Septal Defect with Major Aortopulmonary Collateral Arteries.

[This retracts the article DOI: 10.1155/2021/3483976.].

20-Year Experience With Repair of Pulmonary Atresia or Stenosis and Major Aortopulmonary Collateral Arteries

BackgroundWe have followed a consistent, albeit evolving, strategy for the management of patients with pulmonary atresia or severe stenosis and major aortopulmonary collateral arteries (MAPCAs) that aims to achieve complete repair with low right ventricular pressure by completely incorporating blood supply and relieving stenoses to all lung segments. ObjectivesThe purpose of this study was to characterize our 20-year institutional experience managing patients with MAPCAs. MethodsWe reviewed all patients who underwent surgery for MAPCAs and biventricular heart disease from November 2001 through December 2021. ResultsDuring the study period, 780 unique patients underwent surgery. The number of new patients undergoing surgery annually was relatively steady during the first 15 years, then increased substantially thereafter. Surgery before referral had been performed in almost 40% of patients, more often in our recent experience than earlier. Complete repair was achieved in 704 patients (90%), 521 (67%) during the first surgery at our center, with a median right ventricular to aortic pressure ratio of 0.34 (25th, 75th percentiles: 0.28, 0.40). The cumulative incidence of mortality was 15% (95% CI: 12%-19%) at 10 years, with no difference according to era of surgery (P = 0.53). On multivariable Cox regression, Alagille syndrome (HR: 2.8; 95% CI: 1.4-5.7; P = 0.004), preoperative respiratory support (HR: 2.0; 95% CI: 1.2-3.3; P = 0.008), and palliative first surgery at our center (HR: 3.5; 95% CI: 2.3-5.4; P < 0.001) were associated with higher risk of death. ConclusionsIn a growing pulmonary artery reconstruction program, with increasing volumes and an expanding population of patients who underwent prior surgery, outcomes of patients with pulmonary atresia or stenosis and MAPCAs have continued to improve.

Brain Abscess Drainage in a Case of Tetralogy of Fallot with Pulmonary Atresia with Major Aortopulmonary Collateral Arteries Under Scalp Block

AbstractTetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. There are a few reports of uncorrected TOF patients surviving into adulthood requiring noncardiac surgery. They are commonly susceptible to developing brain abscesses due to paradoxical embolism and lack of pulmonary phagocytic clearance. A 32-year-old man, a known patient of TOF with pulmonary atresia and major aortopulmonary collateral arteries, presented with left side weakness and seizures. He was drowsy with a Glasgow Coma Scale (GCS) of 13/15, heart rate of 72/min, oxygen saturation of 78% with 6 L/min oxygen via face mask, and blood pressure of 90/60 mm Hg with noradrenaline at 0.5 mcg/kg/min. He had central cyanosis, clubbing, and icterus. The left upper and lower limb power was 2/5, whereas it was 5/5 for the right. Cardiovascular examination revealed a grade 3/6 continuous murmur at the base of the heart. No pulmonary abnormality was noted on auscultation. Echocardiography showed large subaortic ventricular septal defect (VSD) with overriding of the aorta, pulmonary atresia, right-sided aortic arch, and ejection fraction of 62%. Magnetic resonance imaging (MRI) of the brain revealed features suggestive of brain abscess. Brain abscess drainage was performed under scalp block with sedation to avoid major hemodynamic fluctuations. The procedure was uneventful, and the patient's power on the left side improved postprocedure. Scalp block with sedation is a suitable alternative to general anesthesia for drainage of brain abscess in TOF patients.

A Deep Dive Into Retroesophageal Major Aortopulmonary Collateral Arteries.

Background: The anatomy of major aortopulmonary collateral arteries (MAPCAs) can be highly variable with regard to number, anatomic origin, course, and relationship to the native pulmonary arteries. Some MAPCAs travel behind the esophagus (retroesophageal) and bronchus before entering the lung parenchyma. The purpose of this paper was to review the anatomy, physiology, and surgical characteristics of retroesophageal MAPCAs. Methods: This manuscript summarizes the data from a series of three papers that have focused on the subject of retroesophageal MAPCAs from our institution over the past ten years. Results: Two-thirds of patients evaluated had a retroesophageal MAPCA identified at surgery. Retroesophageal major aortopulmonary collateral arteries (REMs) were more common with a left arch (77%) compared with a right arch (53%). Of all REMs evaluated, 83% were single supply, 13% were dual supply with an inadequate connection, and 4% were dual supply with an adequate connection. Based on these findings, 96% of retroesophageal MAPCAs were unifocalized. Follow-up catheterization was performed at a median of 17 months after surgery; 75% of unifocalized MAPCAs were widely patent, 20% were patent but stenotic, and 5% were occluded. Conclusions: The data demonstrate that retroesophageal MAPCAs are relatively common and almost always require unifocalization. At mid-term follow-up, 95% of unifocalized MAPCAs were found to be patent.

Outcomes of Surgical Repair of Tetralogy of Fallot: A Comparison Between the Adult and Pediatric Population.

Introduction Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. Early detection and timely treatment have provided successful repair of the anomaly in the developed world. However, in the developing world, there is still a burden of uncorrected TOF patients reaching adulthood. The goal of this study is to determine whether there is any difference in postoperative complications between adult and pediatric populationsfollowing surgical correction for TOF. Methods This study involved all those patients who received primary or secondary surgical repair for TOF in our facility between January 2017 and December 2020. The patients were split according to their age into the pediatric groupif they were under 18 years and the adult groupif they were 18 years or older. Patients with absent pulmonary valve or pulmonary atresia were not included in this study. Patients with large major aortopulmonary collateral arteries (MAPCA) were also excluded from this study. All patients underwent total correction through a median sternotomy approach. The ventricular septal defect was closed with a Bard knitted fiber patch. The right ventricular outflow tract (RVOT) was augmented by excising muscle bands or fibrous bands in the RVOT. If the annulus was smaller than the 3.5 z score, then a transannular patch was done using an autologous pericardium. The main pulmonary artery was augmented in every surgery using an autologous pericardial patch. All patients were shifted to the ICU on the ventilator and were extubated after fulfillment of the extubation criteria. Postoperative complications measured included re-opening, re-intubation, prolonged ventilation (>24 hours), and mortality within the index hospital admission. The clinical data of all patients were prospectively collected and analyzed using the chi-square test and t-test. A p-value of less than or equal to 0.05 was considered significant. Results The total number of patients was 134. This included 83 males (60.1%). A total of 114 patients who were aged below 18 years were included in the pediatric group, and 20 patients aged equal to or more than 18 years were included in the adult group. The mean average perfusion time in minutes in the adult group was 125.8 and in the pediatric group, it was 98.79. Similarly, the mean average of the cross-clamp time was also longer in the adult group at 89.55 minutes versus 69.63 minutes in the pediatric group. Overall, in the adult group, three (15%) patients had postoperative complications, while in the pediatric group, a total of 14 (11.9%) patients had postoperative complications (p = 0.001). However, there was no significant difference in the number of re-openings (8.5% vs. 10%; p = 0.8). The total mortality observed was 16 (11.59%). This included 14 (11.9%) in the pediatric group and two (10%) in the adult group. There was no significant difference between the two groups (p = 0.8). Conclusions Surgical repair of TOF can be performed in both adult and pediatric populations with acceptable outcomes. The mortality rate was found to be slightlygreater in the pediatric population compared to the adults. However, it can be seen that the number of postoperative complications is greater in adults. Further research is needed to optimize outcomes for both pediatric and adult patients with TOF.

Evaluation and comparison of patch materials used for pulmonary arterioplasty in pediatric congenital heart surgery

ObjectiveTo evaluate the long-term performance of the patch materials we have used to augment the pulmonary arterial tree across a wide spectrum of diagnoses and anatomical locations. MethodsRetrospective, single-center review of 217 consecutive pediatric patients at a tertiary referral center from 1993 to 2020 who underwent patch arterioplasty of the pulmonary arterial tree from the pulmonary bifurcation to the distal pulmonary arterial branches. Reintervention data were collected and analyzed. Lesion-specific anatomy and other variables were analyzed as risk factors for reintervention. ResultsThere were 280 total operations performed (217 initial operations and 63 reoperations) and 313 patches used. The patches used were autologous pericardium (166, 53.0%), pulmonary homograft (126, 40.3%), and a heterogeneous group of other materials (21, 6.7%). Overall patient survival was 86.2%, freedom from reoperation was 81.0% and freedom from reintervention (FFR) was 70.6%, with a median follow-up of 13.8 years (interquartile range, 6.3-17.9 years). For all patches, 10-, 20-, and 27-year FFR was 76.6%, 70.6%, and 70.6%, respectively. FFR was similar among all 3 patch type groups (P = .29). Multivariable Cox regression analysis showed that diagnoses of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries and hypoplastic left heart syndrome, patches placed at initial cardiac operation, and increasing number of cardiac operations were risk factors for reintervention. ConclusionsAutologous pericardium and pulmonary homograft patches performed similarly. Although patch type conferred no difference in need for reintervention, other risk factors did exist. Namely, diagnoses of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries and hypoplastic left heart syndrome, patch placement at a patient's first cardiac operation, and increasing number of cardiac operations were risk factors for reintervention.

Retroaortic Innominate Vein in Association With Pulmonary Atresia/Major Aortopulmonary Collaterals

BackgroundRetroaortic innominate vein (RAIV) is an extremely rare anomaly of systemic venous return. The prevalence of RAIV has been estimated to be 0.02% in individuals without congenital heart disease and 0.5% in those with congenital heart disease. Previous studies have demonstrated an association between RAIV and both conotruncal abnormalities and right aortic arch. Our center specializes in surgical procedures for patients with pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs), and we have frequently observed the presence of RAIV in these patients. The purpose of this study was to evaluate the prevalence and anatomic characteristics of RAIV. MethodsThis was a single-center retrospective review of medical records to identify patients with an RAIV. The study period was 2002 through 2022, with approximately 20,000 patients evaluated during this time frame. ResultsA total of 99 patients were identified with an RAIV. Of the 99 patients, 64 (65%) had PA/VSD/MAPCAs. Other diagnoses included tetralogy of Fallot (n = 16), tetralogy of Fallot with pulmonary atresia (n = 3), double outlet right ventricle (n = 4), peripheral pulmonary artery stenosis (n = 3), and other (n = 9). In the cohort of patients with PA/VSD/MAPCAs, 69% had a right aortic arch and 30% had completely absent central pulmonary arteries. ConclusionsThe overwhelming majority (90%) of patients with RAIV had conotruncal abnormalities, with PA/VSD/MAPCAs accounting for the preponderance of cases in our center.

Modified central shunt in pulmonary atresia with diminutive pulmonary arteries.

The commonly used central shunt (i.e. direct connection of the divided main pulmonary artery to the ascending aorta) to facilitate the growth of the diminutive branch pulmonary artery in patients with pulmonary atresia with major aortopulmonary collateral arteries can cause main pulmonary artery distortion, and subsequent disproportional branch pulmonary artery flow and growth. This report describes a novel technique of shunt construction to minimize pulmonary artery torque and tension by using a modified central shunt constructed from autologous pericardium that is anastomosed in an end-to-side fashion to the lateral aspect of the ascending aorta. This shunt will serve as an intermediary conduit to facilitate the main pulmonary artery anastomosis while minimizing torque and tension on the main pulmonary artery. The tension-free connection between the main pulmonary artery and the aorta enabled by the modified central shunt supports proportional pulmonary artery flow and growth in patients with pulmonary atresia, ventricular septal defect, or multiple aortopulmonary collateral arteries and concomitant diminutive native pulmonary arteries.

Clinical deterioration following arterial switch surgery due to extensive aortopulmonary collaterals, unusual condition but worth considering: case report

BackgroundThe occurrence of major aortopulmonary collateral arteries (MAPCAs) is infrequent in patients with D-transposition of great arteries (D-TGA) with intact ventricular septum (IVS). Hemodynamically significant MAPCAs may complicate the postoperative course of these patients after arterial switch operation (ASO).Case presentationWe present a rare case of neonatal D-TGA-IVS associated with extensive MAPCAs. After the ASO, the patient developed pulmonary hemorrhage, chest wall edema, and deterioration of lung compliance with the need for high-frequency ventilation (HFV). The patient also had a significant capillary leak with skin edema, high chest tube drainage, and high peritoneal drainage. Cardiac catheterization revealed extensive MAPCAs supplying the whole lung segments. After the catheter closure of most of these MAPCAs, the patient had clinical improvement.ConclusionsAlthough the occurrence of MAPCAs with D-TGA-IVS is infrequent, clinicians should suspect their presence in cases with unexplained heart failure, pulmonary hemorrhage, or cardiovascular compromise after ASO. Catheter closure of MAPCAs is feasible with an acceptable short-term outcome.

Challenges and outcomes of pregnancy in an uncorrected Tetralogy of Fallot with pulmonary atresia and major aorta-pulmonary collateral arteries (MAPCA): a case report

BackgroundTetralogy of Fallot is a severe type of congenital heart disease (CHD) and one of the leading indirect causes of mortality & morbidity among women with CHD. We came across a rare case of an uncorrected Tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries in pregnancy. Case presentationWe are reporting the challenges in managing a pregnancy of 25-years-old G3 P0110, previous one stillbirth and who was diagnosed to have congenital heart disease during pregnancy following spontaneous abortion.ConclusionsThis case report highlights the role of multidisciplinary care in managing such a high risk case. It also emphasizes the role of cardiac examination of every woman before pregnancy so that definitive treatment or optimization can be done in time for a better outcome.

Influence of the bronchus on the vascular growth of major aortopulmonary collateral arteries.

Patients with major aortopulmonary collateral arteries (MAPCAs) often require additional surgical or catheter intervention after unifocalization (UF) due to stenosis and poor growth. We hypothesized that the UF design influences vascular growth; assessment was based on the passing route related to the bronchus. We enrolled 5 patients with pulmonary atresia (PA), ventricular septal defect and MAPCA who underwent UF and subsequent definitive repair at our institute from 2008 to 2020. Angiography and computed tomography scans were routinely performed before surgical intervention to clarify pulmonary circulation and the relationships between MAPCAs and the bronchus, which revealed peculiar MAPCAs directed to the pulmonary hilum passing behind the bronchus (defined as retro-bronchial MAPCAs; rbMAPCAs). Vascular growth of rbMAPCAs, non-rbMAPCAs and the native pulmonary artery were assessed using the angiograms before and after repair. The angiogram before UF [age 42 (24-76) days, body weight 3.2 (2.7-4.2) kg] showed that the diameter of the original unilateral PA, rbMAPCA and non-rbMAPCA was 19.95 ± 6.65, 20.72 ± 5.36 and 20.29 ± 7.42 mm/m2, respectively (P = 0.917). UF was completed in a single-stage with the placement of modified Blalock-Taussig shunt through median sternotomy at the age of 1.6 (1.0-2.5) months. Angiograms performed 3.0 (1.0-10.0) years after UF completion demonstrated a smaller rbMAPCA diameter at peri-bronchial region (3.84 ± 2.84 mm/m2) compared to the native unilateral PAs (16.11 ± 5.46 mm/m2, P < 0.0001) and non-rbMAPCA (10.13 ± 4.44 mm/m2, P = 0.0103). RbMAPCAs tend to be stenosed at the point where they cross the bronchus and emerge in the middle mediastinum after in situ UF.

Clinical Characteristics of Coronary-to-Pulmonary Artery Fistula in Patients with Pulmonary Atresia and Ventricular Septal Defect.

The existence of a coronary-to-pulmonary artery fistula (CPF) in pulmonary atresia with ventricular septal defect (PAVSD) potentially affects treatment; however, its clinical features have not been comprehensively described due to the disease's rarity. We reviewed 69 cases from 42 studies to reveal the clinical overview of patients with CPF and PAVSD. Among the included patients, the male-to-female ratio was exactly 1:1, and only two patients (3%) exhibited the 22q11.2 microdeletion syndrome. Regarding anatomical features, CPFs originated from the left coronary artery in 65% of patients, and 62% had other major aortopulmonary collateral arteries. Thirty-nine percent of patients had a definitive CPF diagnosis at 0 years of age, whereas 10% were diagnosed in adulthood. Seventy percent underwent catheter angiography to obtain a definitive CPF diagnosis. Ninety-five percent of patients underwent cardiac surgery, and among them, 43% underwent palliative surgery, whereas 52% underwent one-stage repair. Four patients including three adult patients developed cardiac dysfunction due to myocardial ischemia, and three of them exhibited improved cardiac function after the intervention for CPF. Of all the patients, 88% survived and 12% died. The surgical strategy and prognosis were similar to those in PAVSD patients without CPF. This review provides detailed clinical phenotypes that are potentially useful in enhancing the management of patients with this rare disease.